Giovanna Manzi

Right ventricular concentric hypertrophy and clinical worsening in idiopathic pulmonary arterial hypertension

BACKGROUND Because the pathophysiology of idiopathic pulmonary arterial hypertension (IPAH) is an afterload mismatch, wall stress represents the most important feature affecting the overloaded right ventricle (RV). Thus, the RV mass/volume (M/V) ratio may reflect more adequately the suitability of the remodeling pattern in minimizing RV wall stress compared with the lone RV volume. This study investigated the prognostic effect of the RV M/V ratio in IPAH. METHODS Enrolled in our center were 74 therapy-naïve IPAH patients who were prospectively monitored for the presence of clinical worsening (CW). Baseline evaluation included clinical, hemodynamic, and echocardiographic parameters. Cardiac magnetic resonance was used for RV M/V ratio determination. RESULTS During 541 ± 283 days of follow-up, 31 of 74 patients (42%) presented with CW. Actuarial rates of CW were 14%, 28%, and 46%, at 6, 12, and 24 months, respectively. The RV M/V ratio significantly improved the power of the prognostic model based on traditional clinical, hemodynamic, and imaging parameters (area under the curve: 0.74 vs 0.66, respectively; p = 0.01). When the cutoff values of the RV M/V ratio and cardiac index (CI) derived from receiver operating characteristic curve analysis were combined, patients with a low RV M/V ratio/low CI, low RV M/V ratio/high CI, and high RV M/V ratio/low CI showed a 28.8, 8.8, and 6.1 increase in the hazard ratio, respectively, compared with high RV M/V ratio/high CI patients (p = 0.0001). CONCLUSIONS The RV M/V ratio is an independent predictor of prognosis in IPAH and may allow clinicians to better stratify patients with normal CI, identifying at an early stage those patients at higher risk of right heart failure development before hemodynamic instability appears.

Right ventricular dyssynchrony and exercise capacity in idiopathic pulmonary arterial hypertension

Survival in patients with pulmonary arterial hypertension (PAH) is determined by right ventricular (RV) function adaptation to afterload. How altered RV function impacts on exercise capacity in PAH is not exactly known. 104 idiopathic PAH (IPAH) patients aged 52±14 years underwent a diagnostic right heart catheterisation, a comprehensive echocardiography including two-dimensional speckle tracking for RV dyssynchrony evaluation and a cardiopulmonary exercise test. Multivariate analyses were performed to identify independent predictors of peak oxygen uptake (peak V′O2). A first multivariate analysis of only resting haemodynamic variables identified cardiac index, right atrial (RA) pressure and pulmonary arterial compliance as independent predictors, with low predictive capacity (r2=0.31; p<0.001). A second multivariate analysis model which considered only echocardiographic parameters but without RV dyssynchrony, identified RV fractional area change (FAC) and RA area as independent predictors with still low predictivity (r2=0.35; p<0.001). Adding RV dyssynchrony to the second model increased its predictivity (r2=0.48; p<0.001). Repetition of the three multivariate analyses in patients with preserved RVFAC confirmed that inclusion of RV dyssynchrony results in the highest predictive capability of peak V′O2 (r2=0.53; p=0.001). A comprehensive echocardiography with speckle tracking-derived assessment of the heterogeneity of RV contraction improves the prediction of aerobic exercise capacity in IPAH. Speckle tracking assessment of RV dyssynchrony improves the prediction of aerobic exercise capacity in IPAH http://ow.ly/yHyh30bedtD

The importance of right ventricular function in patients with pulmonary arterial hypertension

ABSTRACT Introduction: Pulmonary arterial hypertension (PAH) is a progressive, life-threatening, and incurable disease. Its prognosis is based on right ventricular (RV) function. Therefore, adequate assessment of RV function is mandatory. Areas covered: This article presents the case of a patient with PAH in which the traditional diagnostic approach did not provide a complete assessment of RV function. The authors show how the analysis of other parameters yielded additional information that improved the management of this patient. Expert commentary: Despite current treatments, PAH often worsens due to progressive RV dysfunction. Appropriate assessment of RV function may facilitate the early identification of patients at risk of RV function impairment. More aggressive treatment of PAH might delay progression of the disease. Traditional risk stratification, which is based on New York Heart Association/World Health Organization (NYHA/WHO) functional class evaluation, the 6-minute walk test, and right heart catheterization, proves insufficient in many PAH patients, as it does not provide complete information about RV function. Thus, further parameters are required. Analysis of RV function, in addition to echocardiography and cardiopulmonary exercise testing, may add relevant prognostic information and improve therapy.